Is alternative cardiac surgery an option in adults with congenital heart disease referred for thoracic organ transplantation?

OBJECTIVES: We analysed the outcomes of adults with congenital heart disease (ACHD) referred for thoracic organ transplantation who underwent non-transplant cardiac surgery as an alternative management option. METHODS: Adult patients with congenital heart disease assessed for heart or heart-lung transplant were identified from the departmental database. A retrospective analysis of the medical records, transplant assessment data and surgical notes was carried out. RESULTS: One hundred and twenty-six patients were assessed between January 2000 and July 2011. Non-transplant cardiac surgery was performed in 14 (11%) patients. There were nine males with a median age of 37 years (range 21-42). The patients can be divided into four subgroups [left-sided lesions (n = 4), right-sided lesions (n = 3), systemic right ventricle (n = 5) and Fontan circulation (n = 2)]. Surgical procedures performed were: relief of systemic obstructive/regurgitant lesions ± endocardial fibroelastosis resection (n = 4, three pulmonary vascular resistance >6 Wood units), correction of right-sided regurgitant/stenotic lesions (n = 3), ventricular assist device for patients with a systemic right ventricle (n = 5) and re-fashioning of the Fontan pathway (n = 2). There were two early (5 and 30 days) and three late deaths (64, 232 and 374 days) with a 1-year mortality of 28%. None of the deaths occurred in patients with a two-ventricle circulation and atrio-ventricular concordance. Nine patients are alive at a median of 433 days (range 204-2456). The New York Heart Association class has improved in all survivors by at least one class at 3 and 6 months (P = 0.004 and 0.003). CONCLUSIONS: Alternative cardiac surgery can be undertaken in selected patients with ACHD referred for cardiopulmonary transplantation with a low mortality in patients with two ventricles and a systemic left ventricle. Ventricular assist devices carry a significant mortality in patients with a systemic right ventricle, although this offers a valuable palliation when there are no other options. The medium and long-term results are awaited.